Acrodermatitis Dysmetabolica With Concomitant Acquired Acrodermatitis Enteropathica in a Patient With Maple Syrup Urine Disease
January 2024
in “
JAAD case reports
”
TLDR MSUD patients need careful monitoring of amino acids and zinc to prevent severe symptoms.
A 24-year-old woman with maple syrup urine disease (MSUD) exhibited symptoms of acrodermatitis dysmetabolica (AD) and acquired acrodermatitis enteropathica (AE), including a nonpruritic skin eruption, diarrhea, and hair loss, due to deficiencies in leucine, valine, zinc, and albumin. Despite supplementation, she died from sepsis. This case underscores the need for careful monitoring and balancing of branched-chain amino acids (BCAAs) and zinc levels in MSUD patients to prevent AD and AE-like symptoms.
